CHAPTER X CHAPTER 4 - Cancer et environnement

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A Fig. 4.73 A Sertoli cell tumour. B Sertoli cell tumour mimicking seminoma. B (30%) {2575,2894}. Tumour cells are invariably negative for placental alkaline phosphatase, alpha-fetoprotein, human chorionic gonadotropin. Ultrastructure Charcot-Böttcher crystals, composed of filaments, are rarely seen but are considered to be typical of Sertoli cells. Variants In addition to Sertoli cell tumours NOS two variants are recognized: large cell calcifying Sertoli cell tumour, and sclerosing Sertoli cell tumour. There are not enough data to determine whether the proposed variants such as "lipid rich variant" and "Sertoli cell tumour with heterologous sarcomatous component" {875} warrant separation from the Sertoli cell tumour NOS. Large cell calcifying Sertoli cell tumour (LCCST) Large cell calcifying Sertoli cell tumour (LCCST) can be sporadic, but occur also as parts of the Carney and Peutz- Jeghers syndromes {1391}. Only about 50 cases of this neoplasm have been reported so far. Sporadic tumours account for 60% of cases, whereas the remaining 40% are associated with genetic syndromes or have endocrine disorders {1391}. Endocrine symptoms, including precocious puberty and gynecomastia are found in a significant number of cases. In contrast to Sertoli cell tumours NOS, most patients harbouring LCCST are young and the average age is 16 years. The youngest patient on record was 2 years old. In most cases the tumours are benign, but 20% are malignant. In 40% of cases the tumours are bilateral. Microscopic features of LCCST include nests and cords of relatively large polygonal cells with eosinophilic cytoplasm embedded in myxohyaline stroma. Tumour cells have vesicular and relatively large nuclei and prominent nucleoli, but mitoses are rare. The stroma may be hyalinized, often with abundant neutrophils, and typically shows broad areas of calcification, though a substantial proportion lack calcification. Intratubular spread of the tumour cells is typically found in most cases {366}. Sclerosing Sertoli cell tumour (SSCT) Sclerosing Sertoli cell tumour (SSCT) is rare and less than 20 cases of this variant are recorded {929,2951}. They occur in adults and the average age at the time of diagnosis is 35 years. Most tumours on record are relatively small (0.4-1.5 cm). Microscopically, features of SSCT include small neoplastic tubules surrounded by dense sclerotic stroma. The tubules may be solid or hollow, and may be discrete or anastomosing. Typically the tumours contain entrapped non neoplastic tubules. Differential diagnosis Sertoli cell tumours NOS need to be dis- A B Fig. 4.74 Sertoli cell tumour A Large cell calcifiying variant. Cords and nests of cells in a fibrous stroma with focal ossification. B Large cell calcifiying Sertoli cell tumour. 254 Tumours of the testis and paratesticular tissue
tinguished from Sertoli cell nodules, and Leydig cell tumours, and rete adenomas. Sertoli cell nodules, however, are small, incidentally discovered, non neoplastic lesions composed of aggregates of small tubules lined by immature Sertoli cells and contain prominent basement membrane deposits. The rete adenomas occur within the dilated lumens of the rete testis. Prognosis Most Sertoli cell tumours are benign. Malignant Sertoli cell tumour ICD-O code 8640/3 Epidemiology Malignant Sertoli cell tumour not otherwise specified is rare {1194}. Less than 50 cases have been reported. Age distribution does not differ from that of the benign form, occurring from childhood to old age. Clinical features Some patients present with metastases; most commonly to inguinal, retroperitoneal and/or supraclavicular lymph nodes. Approximately one third has gynecomastia at presentation, but apart from that no specific lesions or syndrome are known to be associated with malignant Sertoli cell tumour. Macroscopy They tend to be larger than the benign counterparts {2894}, usually more than 5 cm but range 2 to 18 cm. The macroscopic appearance may differ from that of the benign tumour by necrosis and haemorrhage. Histopathology Microscopically, the cellular features and Fig. 4.75 Sclerosing type of Sertoli cell tumour. growth patterns are similar to those of the benign counterpart but tend to be more variable within the same tumour and between tumours. The solid, sheet-like growth pattern is often prominent. The nuclei may be pleomorphic with one or more nucleoli, which are usually not very prominent. Mitotic figures may be numerous, and necrosis may occur. A fibrous, hyalinized or myxoid stroma occurs in varying amounts, but is usually sparse. Lymphovascular invasion may be seen. Lymphoplasmacytic infiltration is reported in some cases varying from sparse to pronounced and even with secondary germinal centres. The most important differential diagnoses are classical and spermatocytic seminoma and variants of yolk sac tumour, however granulomatous reactions and intratubular germ cell neoplasia are not present in the surrounding testicular parenchyma. Endometrioid adenocarcinoma and metastases, and among the latter especially adenocarcinomas with pale or clear cytoplasm, as well as melanoma should also be considered. Immunohistochemical staining is helpful in defining the Sertoli cell nature of the tumour but not its malignant potential {1074,1194}. The tumour cells are cytokeratin and vimentin positive and they may also be positive for epithelial membrane antigen. They stain for inhibin A, but usually not very intensely, and they may be S100 positive. They are PLAP and CEA negative. Granulosa cell tumour group Definition Granulosa cell tumours of the testis are morphologically similar to their ovarian counterparts. Two variants are distinguished: adult and juvenile types. Fig. 4.76 Malignant Sertoli cell tumour. A Fig. 4.77 Malignant Sertoli cell tumour. A Solid and tubular components. B Vimentin staining in the tubular component. B Sex cord / gonadal stromal tumours 255
Page 1 and 2: CHAPTER 4X Tumours of the of the Te
Page 3 and 4: TNM classification of germ cell tum
Page 5 and 6: Germ cell tumours P.J. Woodward A.
Page 7 and 8: senting symptom: back or abdominal
Page 9 and 10: A B Fig. 4.03 Germ cell tumours gen
Page 11 and 12: process at all {1524}. In addition,
Page 13 and 14: A B Fig. 4.12 Comparison of morphol
Page 15 and 16: small tumour insufficient to produc
Page 17 and 18: A B C Fig. 4.22 Seminoma. A Pseudog
Page 19 and 20: A B Fig. 4.27 Spermatocytic seminom
Page 21 and 22: Differential diagnoses Differential
Page 23 and 24: Histologic patterns Microcystic or
Page 25 and 26: A Fig. 4.40 Yolk sac tumour. A Pleo
Page 27 and 28: Teratoma Teratomas are generally we
Page 29 and 30: A B C Fig. 4.49 Teratoma. A Teratom
Page 31 and 32: A B Fig. 4.52 Teratoma with somatic
Page 33 and 34: A B Fig. 4.59 Mixed germ cell tumou
Page 35 and 36: A B C Fig. 4.64 Leydig cell tumour.
Page 37: A Fig. 4.69 Androgen insensitivity
Page 41 and 42: ound and have spherical, regularly
Page 43 and 44: Tumours containing both germ cell a
Page 45 and 46: Miscellaneous tumours of the testis
Page 47 and 48: Lymphoma and plasmacytoma of the te
Page 49 and 50: Tumours of collecting ducts and ret
Page 51 and 52: Tumours of paratesticular structure
Page 53 and 54: Epidemiology Nodular mesothelial hy
Page 55 and 56: A B Fig. 4.104 Papillary cystadenom
Page 57 and 58: Fig. 4.109 Desmoplastic small round
Page 59 and 60: Fig. 4.115 Leiomyosarcoma. Coronal,
Page 61 and 62: Secondary tumours C.J. Davis Defini

CHAPTER X CHAPTER 4 - Cancer et environnement

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